Section of Histopathology, Indus Hospital & Health Network, Karachi, Pakistan.
Department of Paediatric Oncology, Indus Hospital & Health Network, Karachi, Pakistan.
Pediatr Blood Cancer. 2022 May;69(5):e29625. doi: 10.1002/pbc.29625. Epub 2022 Mar 6.
Retinoblastoma (RB) tumors having high-risk histopathologic features (HRFs) have an increased risk of metastasis and disease relapse. However, RB has not been studied widely in Pakistan. Therefore, we evaluated the association of clinical, histopathologic, and radiological findings with HRFs in patients with RB who were treated at the Indus Health & Hospital Network in Karachi, Pakistan.
We enrolled treatment-naïve patients with RB who received upfront enucleation from September 2017 to February 2021. We evaluated enucleated eyes with the Intraocular Classification of Retinoblastoma system and classified HRFs as invasion of the anterior chamber, including the iris and ciliary body, or massive invasion of the choroid, sclera, or optic nerve (postlaminar and/or up to the transection line).
Of 117 patients with RB treated at our institution during the study period, 54 received upfront enucleation. Unilateral disease was present in 92.6% of cases. The most frequent disease signs and symptoms included the presence of vitreous seeds (30.6%) and leukocoria (100%), respectively. The most frequent HRFs and radiological findings comprised massive choroidal invasion (15.1%) and anterior chamber enhancement (66.7%), respectively. The majority (62.9%) of patients did not exhibit any HRFs. Female sex, pseudohypopyon, iris neovascularization, buphthalmos, and glaucoma had significant predictive ability for HRF occurrence.
Pseudohypopyon, iris neovascularization, buphthalmos, and glaucoma are important clinical factors that should be taken into consideration before the management of RB. Early recognition of high-risk histopathological and radiological features is essential for appropriate treatment of RB.
具有高危组织病理学特征（HRFs）的视网膜母细胞瘤（RB）肿瘤转移和疾病复发的风险增加。然而，RB 在巴基斯坦并未得到广泛研究。因此，我们评估了在巴基斯坦卡拉奇的 Indus Health & Hospital Network 接受治疗的 RB 患者的临床、组织病理学和影像学发现与 HRFs 的相关性。
我们招募了在 2017 年 9 月至 2021 年 2 月期间接受初次眼球摘除术的未经治疗的 RB 患者。我们使用眼内视网膜母细胞瘤分类系统评估了被摘除的眼睛，并将 HRFs 分类为前房侵犯，包括虹膜和睫状体，或脉络膜、巩膜或视神经的大量侵犯（后板层和/或至横断线）。
在研究期间，我们机构共治疗了 117 例 RB 患者，其中 54 例接受了初次眼球摘除术。单侧疾病占 92.6%。最常见的疾病体征和症状分别为玻璃体内种子的存在（30.6%）和白瞳症（100%）。最常见的 HRFs 和影像学表现分别为广泛的脉络膜侵犯（15.1%）和前房增强（66.7%）。大多数（62.9%）患者没有任何 HRFs。女性、假性前房积脓、虹膜新生血管形成、牛眼和青光眼对 HRF 发生具有显著预测能力。
假性前房积脓、虹膜新生血管形成、牛眼和青光眼是 RB 管理前应考虑的重要临床因素。早期识别高危组织病理学和影像学特征对于 RB 的适当治疗至关重要。
